Journal
THROMBOSIS AND HAEMOSTASIS
Volume 104, Issue 3, Pages 531-535Publisher
SCHATTAUER GMBH-VERLAG MEDIZIN NATURWISSENSCHAFTEN
DOI: 10.1160/TH09-12-0860
Keywords
ADAMTS13; alternative splicing; intron retention; TTP; CUB domain
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Funding
- Intramural NIH HHS [Z01 BC005598-18] Funding Source: Medline
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Although ADAMTS13, the von Willebrand factor (VWF)-cleaving protease, is expressed in a range of tissues, the physiological significance of tissue-specific ADAMTS13 alternative splicing isoforms have yet to be clarified. Screening a panel of human tissues and cell lines revealed a spliced ADAMTS13 transcript in hepatic stellate cells and a hepatoma cell line that retains the 25(th) intron.A nonsense codon within the intron truncates the protease, which gains 64 novel amino acids in lieu of both CUB domains. This isoform, while retaining VWF-cleaving capability, accumulates intracellularly and its biological inaccessibility may prevent its participation in regulating haemostasis and other physiologic functions.
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